Pekmezovic Tatjana, Svetel Marina, Maric Jelena, Dujmovic-Basuroski Irena, Dragasevic Natasa, Keckarevic Milica, Romac Stanka, Kostic Vladimir S
Institute of Neurology, Clinical Centre of Serbia, School of Medicine, University of Belgrade, Dr Subotica 6, 11000, Belgrade, Serbia.
Eur J Epidemiol. 2007;22(8):523-6. doi: 10.1007/s10654-007-9157-7. Epub 2007 Jul 25.
The objective of this study was to estimate probability of survival of Huntington's disease (HD) patients in Serbia as a function of CAG repeat length and selected demographic variables. This follow-up study was carried out at the Institute of Neurology, Clinical Centre of Serbia, Belgrade, 1982-2004. The study group consisted of 112 HD patients. The significant inverse correlation was found between CAG repeat length and age at onset of HD (r = -0.732, P = 0.001) and age at death (r = -0.760, P = 0.001). The cumulative probabilities of survival in a five, ten, fifteen, and twenty-years' period were 90.9, 63.2, 10.3 and 4.5%, respectively. Higher survival probabilities were registered in female patients, as well as in those with older age at onset and lower number of CAG repeat length (</=46). The Cox regression analysis showed that significantly poorer outcome of HD in our population was related to younger age at onset (HR-hazard ratio = 1.9; P = 0.047), and larger CAG numbers (HR = 2.4; P = 0.071). The female sex was statistically significantly associated with longer survival (HR = 0.4; P = 0.007). These data might be of some importance for further exploration of natural history and prognosis of HD.
本研究的目的是评估塞尔维亚亨廷顿舞蹈症（HD）患者的生存概率，该概率是CAG重复序列长度和选定人口统计学变量的函数。这项随访研究于1982年至2004年在贝尔格莱德塞尔维亚临床中心神经病学研究所进行。研究组由112名HD患者组成。研究发现CAG重复序列长度与HD发病年龄（r = -0.732，P = 0.001）和死亡年龄（r = -0.760，P = 0.001）之间存在显著的负相关。在五年、十年、十五年和二十年期间的累积生存概率分别为90.9%、63.2%、10.3%和4.5%。女性患者以及发病年龄较大且CAG重复序列长度较低（≤46）的患者的生存概率更高。Cox回归分析表明，在我们的人群中，HD预后明显较差与发病年龄较小（风险比HR = 1.9；P = 0.047）和CAG重复序列数量较多（HR = 2.4；P = 0.071）有关。女性在统计学上与更长的生存期显著相关（HR = 0.4；P = 0.007）。这些数据可能对进一步探索HD的自然史和预后具有一定重要性。
